Department of Ophthalmology and Visual Sciences, School of Medicine and Public Health, University of Wisconsin-Madison, 1300 University Ave, MSC 3385, 53706, Madison, WI, USA,
Adv Exp Med Biol. 2014;801:267-74. doi: 10.1007/978-1-4614-3209-8_34.
Assembly of sub-lytic C5b-9 membrane attack complexes (MAC) on the plasma membrane of retinal pigment epithelial cells contributes to the pathogenesis of age-related macular degeneration. C5b-9 pores induce calcium influx, which activates signaling pathways that compromise cell function. Mechanisms that limit sub-lytic MAC activity include: cell surface complement regulatory proteins CD46, CD55, and CD59 that inhibit specific steps of MAC formation; elimination of assembled MAC by exocytosis of membrane vesicles or by endocytosis and subsequent lysosomal degradation; and rapid resealing of pores by the exocytosis of lysosomes. Aging in the post-mitotic retinal pigment epithelium is characterized by the accumulation of cellular debris called lipofuscin, which has also been associated with retinal diseases such as age-related macular degeneration. Lipofuscin has been shown to activate complement components both in vitro and in vivo, suggesting that it could contribute complement-mediated dysfunction in the retinal pigment epithelium. Here, we discuss emerging evidence that vesicular trafficking in the retinal pigment epithelium is critical for efficient removal of MAC from the cell surface and for limiting inflammation in the outer retina.
亚溶细胞 C5b-9 膜攻击复合物(MAC)在视网膜色素上皮细胞的质膜上的组装导致年龄相关性黄斑变性的发病机制。C5b-9 孔诱导钙离子内流,激活信号通路,损害细胞功能。限制亚溶细胞 MAC 活性的机制包括:细胞表面补体调节蛋白 CD46、CD55 和 CD59,它们抑制 MAC 形成的特定步骤;通过膜小泡的胞吐作用或内吞作用和随后的溶酶体降解来消除组装的 MAC;以及通过溶酶体的胞吐作用快速封闭孔。有丝分裂后视网膜色素上皮的衰老表现为称为脂褐素的细胞碎片的积累,脂褐素也与年龄相关性黄斑变性等视网膜疾病有关。脂褐素已被证明在体外和体内都能激活补体成分,这表明它可能导致视网膜色素上皮中补体介导的功能障碍。在这里,我们讨论了新出现的证据,即视网膜色素上皮中的囊泡运输对于从细胞表面有效去除 MAC 和限制外视网膜炎症至关重要。